CD4-CD8- T-cell polymyositis in a patient with chronic active Epstein-Barr virus infection

Abstract

We describe a 17-year-old woman with chronic active Epstein-Barr virus infection (CAEBV), who developed EBV+CD4-CD8- T-cell polymyositis. At 14 years of age, CAEBV was diagnosed with fever, cytopenia, liver dysfunction, and hepatosplenomegaly. Despite the transient remission of interferon-α therapy, migratory lesions emerged in back and extremities. MRI indicated polymyositis. Biopsy specimens revealed intramuscular infiltration of CD3+, CD4-, CD8-, CD56-, and EBV-encoded RNA 1+ cells. Circulating CD4-CD8-Vδ2/Vγ9 cells increased. γδT-cells contained 20-200 times higher EBV-DNA (2 x 104 copies/μgDNA) than αβT-cells or NK-cells. The ominous polymyositis might denote the musculotropic invasion of EBV+γδT-cell lymphoproliferative disease as a consequence of CAEBV. © 2002 Wiley-Liss, Inc.